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PsAPASH syndrome — Clinical Guidelines

Overview

Generalized pustular psoriasis (GPP) is a rare, potentially life-threatening auto-inflammatory disease characterized by sterile pustules on erythematous skin 2 4. There are currently no universally accepted treatment guidelines 2.

Diagnosis

No diagnostic criteria or recommended tests are detailed in the provided abstracts.

Management

First-line treatments include acitretin, cyclosporine, methotrexate, and infliximab 4.

Biologics targeting IL-17 or IL-23 have shown efficacy, including ixekizumab, secukinumab, brodalumab, guselkumab, and ustekinumab 4.

IL-36 inhibitors appear to achieve higher response rates earlier (40% GPPASI75 and 55% GPPGA(0,1) response within 2 weeks) compared to IL-17, TNF-α, and IL-23 inhibitors 2.

IL-17, TNF-α, and IL-23 inhibitors show a gradual increase in response rates up to 12 weeks 2.

Special Populations

Recommendations for the management of pediatric psoriasis are available 1.

Key Recommendations

IL-36 inhibitors achieve high response rates in GPP within 2 weeks, outperforming other biologics 2. (Evidence: Moderate)

Established therapies such as acitretin, cyclosporine, methotrexate, and infliximab are considered first-line treatments for GPP 4. (Evidence: Moderate)

Biologics targeting IL-17 or IL-23 are emerging treatment options for GPP 4. (Evidence: Moderate)

References

1 Vicente A, Pérez-Ferriols A, Batalla A, García-Fernandez L, Pérez B, Eiris N et al.. [Translated article] Consensus Statement From the Spanish Academy of Dermatology and Venereology (AEDV) Psoriasis Working Group (SWG) and Pediatric Working Group (PWG) on the Management of Pediatric Psoriasis. Actas dermo-sifiliograficas 2025. link 2 Chen BL, Liu QW, Dong XW, Bai YP. Biologics for generalized pustular psoriasis: a systematic review and single-arm meta-analysis. Frontiers in immunology 2024. link 3 Carrascosa JM, Puig L, Romero IB, Salgado-Boquete L, Del Alcázar E, Lencina JJA et al.. [Translated article] Practical Update of the Guidelines Published by the Psoriasis Group of the Spanish Academy of Dermatology and Venereology (GPs) on the Treatment of Psoriasis With Biologic Agents: Part 2-Management of Special Populations, Patients With Comorbid Conditions, and Risk. Actas dermo-sifiliograficas 2022. link 4 Kearns DG, Chat VS, Zang PD, Han G, Wu JJ. Review of treatments for generalized pustular psoriasis. The Journal of dermatological treatment 2021. link

PsAPASH syndrome